Devita, Hellman, and Rosenberg's Cancer

LWBK1006-26 LWW-Govindan-Review December 12, 2011 19:29

370 DeVita, Hellman, and Rosenberg’s CANCER: Principles and Practice of Oncology Review

ANSWERS

Answer 26.1. The answer is D.

Patients with Li-Fraumeni syndrome have an increased risk for develop-

ing soft tissue sarcomas. Neurofibromatosis type I increases the risk for

the development of malignant peripheral nerve sheath tumors. Patients

with retinoblastoma also have an increased risk for developing soft tissue

sarcomas.

Answer 26.2. The answer is B.

Radiation-induced sarcomas usually occur 10 to 30 years after exposure

to radiation. The most common cancers for which radiation was adminis-

tered include breast and cervical cancer, and lymphoma. Most radiation-

induced sarcomas are osteogenic sarcoma or MFH, and the prognosis is

poor.

Answer 26.3. The answer is A.

Ewing’s sarcoma is associated with five known cytogenetic abnormalities,

includingt(11; 22) (q24; q12). Synovial sarcoma is associated witht(x;

18) (p11; q11), whereas myxoid liposarcoma is associated witht(12; 16)

(q13; p11). Alveolar rhabdomyosarcoma is associated witht(2; 13) (q35;

q14).

Answer 26.4. The answer is D.

The current standard of care for the treatment of osteosarcomas is neoad-

juvant chemotherapy, followed by limb-sparing resection and adjuvant

chemotherapy. The percentage of tumor cell necrosis on pathology from

the resection gives an idea of prognosis and helps determine the adju-

vant chemotherapy regimen. Surgery alone, or definitive radiation, offers

lower chances of success.

Answer 26.5. The answer is D.

The Memorial Sloan Kettering Cancer Center (MSKCC) nomogram

for sarcoma-specific mortality incorporates the following variables: age,

tumor size, depth, histology, grade of tumor, and site. Zero points are

assigned for the low-risk features (age 16, size≤5 cm, superficial depth,

fibrosarcoma histology, low grade and upper extremity site). A sum of the

points assigned for each variable helps predict the risk of sarcoma-specific

death.

Answer 26.6. The answer is B.

Stage IV disease includes the presence of nodal or distant metastases. The

prognosis of patients with small (<5 cm) high-grade soft tissue sarcomas

without metastases is similar to that of other patients with stage II disease.

Stage I disease includes only low-grade tumors. Staging of soft tissue